Pemphigus vulgaris is a rare autoimmune disease that causes blistering of the skin and mucous membranes of the mouth, throat and genital area.
The blisters may vary in size, but are thin-walled and fragile. They break easily and leave areas of raw skin that are painful and susceptible to infection. The blisters usually develop in the mouth at first. In some patients, other areas of the body may be affected at a later stage. Sometimes, large areas of skin peel off at just the rub of a finger. The condition may develop at any age, although the usual age of disease onset is in adults between 50 and 60 years.
This condition is diagnosed by the characteristic clinical appearance, with a skin biopsy from the affected area of the mucous membrane of the mouth. Antibody testing using direct and indirect immunofluorescence is also a useful tool to confirm the diagnosis.
There is no cure for pemphigus vulgaris, but symptoms can be kept under control with the use of corticosteroids or immunosuppressants. General treatment may include:
Antibiotic and antifungal treatment, either for existing infections or as prophylaxis
Intravenous nutrition if there are oral ulcers too severe to allow adequate feeding
Anesthetic mouthwashes or lozenges to control the mouth pain
Painkillers for severe pain
In most cases, pemphigus vulgaris is initially treated with corticosteroids for a few weeks or months to suppress the autoimmune reaction. This helps to heal existing blisters and to prevent the formation of new ones. It usually takes approximately three weeks for new blisters to stop forming, while about eight weeks are required for existing blisters to heal. The treatment is usually taken in tablet form, although injections and creams may also be used. To lower the risk of side effects, the corticosteroid dose is gradually reduced, meanwhile adding another immunosuppressant to replace its action. It may take some time for the best dose to be established. A balance needs to be found between the control of symptoms and the limitation of adverse side effects. It may be possible to eventually stop treatment in a few patients. Most individuals, however, require treatment on an ongoing basis, in order to prevent flare-ups of the condition.
Steroids may cause several unpleasant and even dangerous side effects when used over the long term. These include the following:
Easily bruised and thinned-out skin
Susceptibility to infection
As the dose is gradually reduced, it should be possible to limit these side effects, although osteoporosis can be an ongoing problem.
Once symptoms are under control, another immunosuppressant may be used in combination with the low-dose corticosteroid. Examples include cyclosporine, cyclophosphamide, and azathioprine. These medications may also increase the risk of contracting infection, which is why certain precautions should be taken. These include avoiding close contact with people who have an infection or have been exposed to one, avoiding crowded places and informing your doctor of any symptoms that could indicate infection arise, such as fever.
In cases where a steroid and immunosuppressant fail to bring symptoms under control, another medication may be added to the treatment regimen. However, these treatments are not widely available. Some of these medications are described below:
Rituximab: This helps to stop the autoantibodies that are targeting epidermis cells
Tetracycline and dapsone: These are antibiotics that modify immune system activity
Plasmapheresis: Here, the blood is run through a machine that eliminates the autoantibodies directed against epidermis cells
Immunoglobulin therapy: Normal antibodies that modify the immune system on a temporary basis are administered intravenously
It may be very distressing for people to find out that they have a rare and serious disease. This can trigger feelings of distress, bewilderment, loneliness and fear. A good way to start coping with it is to find out as much information as possible about the condition. To help with this, there are many support groups, which provide a good starting point. They also offer support to people with the condition to help them manage the stress and the pain of the unpredictable remissions and exacerbations.
Other recommendations that may be helpful to manage the condition include the following:
Avoiding activities that could cause the skin to become damaged, such as contact sports
Using a soft toothbrush
Avoid crisp, hard, hot or spicy foods
Taking painkillers, particularly prior to eating or brushing the teeth
Maintaining good oral and skin hygiene
Talcum powder used liberally to prevent the skin sticking to the bedsheets
Frequently changing and laundering towels, sheets and body linen in hot water to prevent secondary infection
seeking medical attention as soon as any symptoms of infection develop
Using exercise and relaxation techniques as ways of improving coping mechanisms
Attending a pain management course
Reviewed by Dr Liji Thomas, MD. References